Faculty Bibliography

A 48-year-old woman with a history of chronic intranasal cocaine abuse presented with unilateral proptosis associated with severe visual loss from optic neuropathy in the right eye. Imaging showed extensive bone and soft tissue destruction in the paranasal region and an orbital mass. Initial biopsies suggested a low-grade neoplasm. The correct diagnosis was established only on repeat biopsy, which revealed marked pleomorphism and nonspecific chronic inflammation with irregular collagen bundles containing thick-walled blood vessels. This case emphasizes that intranasal cocaine abuse may clinically, radiographically, and histopathologically mimic a neoplasm or a necrotizing vasculitis.

Hedgehog signaling is often activated in tumors, yet it remains unclear how GLI2, a transcription factor activated by this pathway, acts as an oncogene. We show that GLI2 is a pleiotropic oncogene. The overexpression induces genomic instability and blocks differentiation, likely mediated in part by enhanced expression of the stem cell gene SOX2. GLI2 also induces transforming growth factor (TGF)B1-dependent transdifferentiation of foreskin and tongue, but not gingival fibroblasts into myofibroblasts, creating an environment permissive for invasion by keratinocytes, which are in various stages of differentiation having downregulated GLI2. Thus, upregulated GLI2 expression is sufficient to induce a number of the acquired characteristics of tumor cells; however, the stroma, in a tissue-specific manner, determines whether certain GLI2 oncogenic traits are expressed

OBJECTIVE: To determine the reliability of the House-Brackmann facial nerve grading scale in the setting of differential function across its branches. STUDY DESIGN: Prospective. SUBJECTS AND METHODS: Eleven physicians with different levels of clinical experience and three upper-level medical students were provided with digital video clips of 11 patients with differential facial nerve functioning, and asked to report facial nerve function as a traditional global score and as a regional score on the basis of the House-Brackmann scale for the forehead, eye, nose, and mouth. Agreements between the traditional global score and the regional scores, as well as inter-rater agreement, were analyzed. RESULTS: In patients with variable facial weakness, a single House-Brackmann score did not fully communicate facial function. The single House-Brackmann score most strongly correlated with the regional scoring of the nose/midface (59%), followed by the mouth (51%), eye (48%), and forehead (35%). Overall inter-reader reliability was relatively strong for the midface (kappa = 0.503) and global scores (kappa = 0.541), followed by the mouth (k = 0.419), the forehead (k = 0.330), and the eye (k = 0.302). There was a marked tendency for reader agreement to increase among those with more clinical experience. CONCLUSION: Regional assessment using the House-Brackmann grading scale more fully communicates facial function and increases in reliability with experience

BACKGROUND: In the past, surgeons believed that in order to eradicate regional disease, a radical or modified radical neck dissection was necessary. An evolution in surgical principles and the popularization of primary chemoradiation has raised the questions regarding the role of neck dissection and the extent of neck dissection following therapy. The aim of this study was to determine the efficacy of selective neck dissection (SND) for patients with N2 or N3 disease following treatment with primary radiation therapy or chemoradiation. METHODS: A retrospective review of 58 patients with stage III or IV head and neck squamous cell carcinoma was conducted. The primary sites included base of tongue (n = 15), hypopharynx (n = 12), tonsil (n = 16), larynx (n = 11), and unknown primary (n = 4). Definitive treatment consisted of either concomitant chemoradiation (67.2%) or external beam radiation therapy (32.8%). In the monotherapy group, all patients received a total curative dose of 66 to 72 Gy in once-daily fractions of 180 to 200 cGy. The combined chemoradiation group received a similar radiation schedule and a 4-day continuous infusion of cisplatin (20 mg/m(2)/day) and 5-fluorouracil (1000 mg/m(2)/day). A planned SND of levels II to IV was performed on all the patients 3 to 6 weeks after completion of definitive medical therapy. RESULTS: Seventy neck dissections were performed on 58 patients with advanced neck disease following radiation or chemoradiation. The median time of follow-up was 34 months (range, 9-71 months) following the neck dissection. Pathologically, 22.4% (13/58) of the patients had viable tumor cells identified in the neck dissection specimen. Seventy-two percent of the patients are currently alive, and 28% died as a result of distant disease, local or regional recurrence, or other causes. Of patients who died from distant disease, 11% had pathological evidence of residual tumor cells identified in the neck dissection specimen. Of patients who died from local or regional disease, 50% had pathological evidence of residual tumor cells identified in the neck dissection specimen. CONCLUSION: The rate of regional recurrence following SND is similar to reported rates following modified/radical neck dissection. This suggests that SND provides an appropriate surgical option for advanced neck disease in select patients following adjuvant therapy.

OBJECTIVE:To characterize the incidence and pattern of neural invasion (NI) in patients with cancers of the paranasal sinuses and anterior skull base. DESIGN/METHODS:Retrospective study. SETTING/METHODS:A tertiary referral cancer center. PATIENTS/METHODS:The study included 208 patients with cancer of the paranasal sinuses. Patients with brain invasion or neurogenic tumors were excluded. MAIN OUTCOME MEASURE/METHODS:Analysis of clinical and pathologic data on patients with cancer of the paranasal sinuses. RESULTS:Forty-one specimens (20%) had evidence of NI. Sinonasal undifferentiated, adenoid cystic, and squamous cell carcinoma had a high propensity for NI, whereas melanoma and sarcoma rarely invaded nerves. Intraneural invasion was found in 32% of these cases, and 34% invaded more than 1 cm distal to the tumor. Neural invasion was associated with a high rate of positive margins, maxillary origin, and previous surgical treatment (P < .04) but not with stage, orbital invasion, or dural invasion. Patients with NI were more likely to undergo adjuvant radiotherapy (P = .003), which significantly improved survival in patients with minor salivary gland carcinomas (P = .04). Multivariate analysis showed that pathologic evidence of NI was not an independent predictor of outcome. CONCLUSIONS:Paranasal carcinomas have high propensity for NI, whereas melanoma and sarcoma rarely invade nerves. Patterns of NI include both perineural and intraneural invasion. Neural invasion is associated with positive margins, maxillary origin, and previous surgery.